In most patients with cystic fibrosis (CF), pulmonary symptoms are the first sign of presentation. Another predominant feature is maldigestion, frequently starting in early childhood. Pancreatic exocrine dysfunction causes maldigestion, characterised by failure to thrive, diarrhoea, hypoproteinaemia, oedema and anaemia. Dermatitis may be the initial manifestation in just a few patients with CF. We report on a patient presenting with refractory dermatitis resembling acrodermatitis enteropathica as the first sign of CF at the age of 4 months, but without pulmonary symptoms. Laboratory findings demonstrated anaemia, hypoproteinaemia and zinc deficiency. Peroral zinc substitution resulted in resolution of the dermatitis within a few days. A further work-up revealed a positive sweat test and homozygosity for the deltaF-580 mutation.
Conclusion: in rare cases, acrodermatitis enteropathica-like dermatitis is the first symptom of cystic fibrosis.